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Increased accumulation of Staufen in nucleus contributes to C9ALS/FTD pathology

Title
Increased accumulation of Staufen in nucleus contributes to C9ALS/FTD pathology
Alternative Title
핵 속에 축적된 Staufen 단백질이 C9ALS/FTD 병리 기전에 기여
Author(s)
Eun Seon Kim
DGIST Authors
Kim, Eun SeonKim, Ho MinLee, Sung Bae
Advisor
이성배
Co-Advisor(s)
Ho Min Kim
Issued Date
2019
Awarded Date
2019-08
Type
Thesis
Description
ALS/FTD, C9ORF72, DPR, Staufen, Drosophila, 루게릭 병, 전두측두엽 치매, 초파리
Table Of Contents
Abstract i
List of contents ii
List of figures iii

I. INTRODUCTION 1
1.1 C9ORF72 expansion causes ALS/FTD pathology 1
1.2 Arginine-rich DPR proteins mainly contribute to C9ALS/FTD pathology 2
1.3 Pathogenic role of Staufen remains elusive in C9ALS/FTD pathology 3

II. MATERIALS AND METHODS 5

III. RESULTS 15
3.1 Arginine-rich DPR proteins increase nuclear accumulation of Staufen 15
3.2 Increased accumulation of nuclear Staufen partially co-localizes with heterochromatin 28
3.3 Increased nuclear accumulation of Staufen is mRNA dependent 34
3.4 Loss of Staufen rescues poly(PR)-mediated toxicity 43

IV. DISCUSSION 47

V. REFERENCES 49

VI. SUMMARY (국문요약) 53
URI
http://dgist.dcollection.net/common/orgView/200000218831

http://hdl.handle.net/20.500.11750/10448
DOI
10.22677/thesis.200000218831
Degree
Master
Department
Department of Brain and Cognitive Sciences
Publisher
DGIST
Related Researcher
  • 이성배 Lee, Sung Bae
  • Research Interests Cellular mechanism of neurodegenerative diseases; Neuronal maintenance and remodeling; 퇴행성 뇌질환의 세포기전; 신경계 유지 및 리모델링 연구
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